Table 2

List of conditions considered to be complex with special risks and complications

Usual nameCommon synonyms/categories
*Surgery can be difficult in adults. Intervention requires specialist experience.
Absent left and right atrioventricular connectionMitral atresia, tricuspid atresia
Anomalous pulmonary venous connection (total or partial)
Aortic valve or aortic root disease requiring intervention in childhood or adolescenceCongenital aortic stenosis or regurgitation, aorto-left ventricular fistula, sinus of Valsalva fistula or aneurysm
Atrioventricular septal defects (partial and complete)Primum ASD, atrioventricular canal, endocardial cushion defects
Coarctation of aorta
Common arterial trunkTruncus arteriosus, hemitruncus
Common atriumSingle atrium. Often associated with laevocardia, anomalies of systemic veins
Transposition of great arteriesd-transposition, TGA, VA discordance
Congenitally corrected transpositionL-transposition, AV and VA discordance)
Congenital coronary artery anomalies, fistulae (unless incidental finding)
Cor triatriatum, left and right sidedDivided atrium
Cyanotic congenital heart disease—all types
Double outlet left ventricle
Double outlet right ventricleTaussig-Bing anomaly
Ebstein’s malformation
Hypoplastic left ventricle
Hypoplastic right ventriclePulmonary atresia with intact ventricular septum
Infundibular stenosisRight ventricular outflow obstruction
Mitral valvar anomaly (congenital)Parachute mitral valve, clefts, absent leaflet, Shone’s syndrome
Functionally single ventricleSingle ventricle (double inlet ventricle, common ventricle)
Persistent ductus arteriosus*
Pulmonary atresia with ventricular septal defectPulmonary atresia with congenital systemic collaterals. Tetralogy of Fallot with pulmonary atresia
Pulmonary hypertension complicating congenital heart diseaseEisenmenger’s syndrome, and residual postoperative pulmonary hypertension after defect or duct closure
Pulmonary valvar regurgitation or stenosis (moderate or greater), including absent pulmonary valve
Subvalvar or supravalvar aortic stenosis
Tetralogy of Fallot
Valved conduitsUsually right sided—homografts, dura, fascia, Carpentier Edwards, valveless, pericardial
Ventricular septal defect (complex)Includes ventricular septal defects with aortic regurgitation, absent leaflets, coarctation, mitral valve disease, straddling tricuspid or mitral valve, subaortic stenosis, two chambered right ventricle
Other anomalies of atrioventricular connection, for example, criss-cross heart, upstairs-downstairs heart
Heterotaxy syndromesIsomerism of atrial appendages, right or left